Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This clinicopathologic study of patients with chronic graft-versus-host disease (GVHD) after allogeneic marrow transplantation emphasizes the most prominent feature of the syndrome, the cutaneous aspects, and describes the ophthalmic-oral sicca syndrome with sialoadenitis and the neurologic findings. Chronic cutaneous GVHD affected 19 of 92 recipients surviving 150 days or more. In 6 patients chronic GVHD presented as a continuation of acute GVHD; in 8 it occurred after the resolution of acute GVHD; and in 5 it arose without preceding acute GVHD, ie, de novo late onset. Two cutaneous types were distinguished. The generalized type affected 16 patients and ran a progressive course resulting in late complications of poikiloderma, diffuse dermal and subcutaneous fibrosis, and contractures. Microscopically, it resembled generalized morphea and lupus erythermatosus hypertrophicus et profundus. The local type affected 3 patients with a more variable picture of poikiloderma, dermal sclerosis, and contractures. Microscopically, it resembled lupus of erythematosus profundus and scleroderma. Guidelines for defining and subclassifying chronic cutaneous GVHD are proposed.
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PMID:Chronic cutaneous graft-versus-host disease in man. 2 21

A review of inflammatory subcutaneous disease revealed that lymphoid follicles are observed frequently in lupus panniculitis. They are found rarely in other lymphocytic inflammatory diseases involving the panniculus, such as morphea, erythema nodosum, and erythema induratum. The lymphoid follicle may be an indication of immunoreactive and connective tissue disease, and cases should be evaluated with this in mind.
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PMID:Lymphoid follicles in subcutaneous inflammatory disease. 43 65

Secondary mucinosis is a common finding in connective tissue diseases, especially in lupus erythematosus and dermatomyositis, but is seen only rarely in morphea. We report the case of a 9-year-old boy who presented with linearly arranged, flesh-colored to erythematous, indurated, very tender plaques on his right arm. He had similar lesions on his midchest and upper back. Histopathology revealed the characteristic findings of morphea and mucin deposition between thickened collagen bundles. This is an unusual case of linear morphea with hyaluronic acid deposition.
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PMID:Linear morphea with secondary cutaneous mucinosis. 147 50

The term fasciitis-panniculitis syndrome (FPS) is proposed as a novel compilation encompassing several disorders, common to which is subcutaneous induration caused by cicatrizing fasciitis as well as septal and lobular panniculitis and perimysial fibrosis. Included herein are Shulman's eosinophilic fasciitis, morphea profunda, lupus profundus, venous lipodermatosclerosis, toxic oil syndrome, altered tryptophane-related eosinophilic myositis, graft-versus-host reaction, and fasciitis reactive to subjacent basal cell carcinoma. FPS should be differentiated from scleroderma, which primarily affects the dermal structures and in which arterioles are injured. In contrast, vasculopathy of the subcutaneous medium-sized veins accompanies the hypodermal lesions of FPS. The importance of recognizing and grouping these disorders lies in their different histopathology, characterization as reactive phenomena, enhanced responsiveness to treatment, and better prognosis than scleroderma. In view of the excellent prognosis of FPS, steroid treatment is not warranted. Long-term therapy with cimetidine appears to benefit the majority of patients.
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PMID:The fasciitis-panniculitis syndrome: clinical spectrum and response to cimetidine. 157 May 16

The extent of eosinophils in skin biopsy and peripheral blood specimens of patients with lupus panniculitis and morphea profunda was determined by studying 37 biopsies from 33 patients with lupus panniculitis and 55 biopsies from 53 patients with morphea profunda. Specimens from 8 (24%) of 33 patients with lupus panniculitis showed eosinophils, and 13 (25%) of 53 patients with morphea profunda had eosinophils in the subcutaneous tissue. In all cases, the diagnosis of lupus panniculitis or morphea profunda was established on the basis of other, more characteristic histologic features; the presence of eosinophils was incidental and not a diagnostic criterion. Occasional cases of lupus panniculitis or morphea profunda had numerous eosinophils. However, in most of the specimens, eosinophils were absent or observed in small numbers. Only 3% of patients with lupus panniculitis showed an increase in the number of eosinophils in the peripheral blood, whereas 47% of patients with morphea profunda had peripheral eosinophilia. The degree of hyaline necrosis in lupus panniculitis or of sclerosis in morphea profunda did not correlate with the number of eosinophils present in the biopsy specimen or peripheral blood.
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PMID:Eosinophils in lupus panniculitis and morphea profunda. 191 6

A women had annular lesions of subacute cutaneous lupus erythematosus that slowly resolved and were replaced by plaques of morphea. The immunologic implications of this unique transitional case of subacute cutaneous lupus erythematosus to morphea are discussed.
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PMID:Subacute cutaneous lupus erythematosus lesions progressing to morphea. 222 33

Biopsy specimens from 185 patients with 52 different skin disorders were investigated by indirect immunofluorescence staining for the presence of HLA-DR bearing keratinocytes and their association with an underlying inflammatory infiltrate and in particular with activated (HLA-DR-positive, Leu-4-positive) T lymphocytes. HLA-DR expression on keratinocytes was demonstrated in 38 dermatoses, including lymphocytic vasculitis, lupus erythematosus, morphea, vitiligo, lichen planus, cutaneous T-cell lymphoma, various infectious dermatoses, allergic contact dermatitis, granulomatous dermatoses, Sweet's syndrome, lichen sclerosus and erythema nodosum. In 27 of these this had not previously been reported. Occurrence of HLA-DR on keratinocytes was invariably linked to the presence of a lymphocytic infiltrate containing numerous activated T-cells (Leu-4 +, HLA-DR +) whereas such infiltrates were not accompanied by HLA-DR expression on keratinocytes in all the dermatoses investigated, as in pseudolymphoma and erythema anulare centrifugum. However, HLA-DR positive keratinocytes were consistently absent in skin disorders lacking any significant lymphocytic infiltration (e.g. leukocytoclastic vasculitis, bullous autoimmune dermatoses, genodermatoses and mastocytosis). Although it has been suggested that HLA-DR-positive keratinocytes are involved in various immune responses of the skin, their exact functional significance is, as yet, unknown.
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PMID:HLA-DR expression on keratinocytes is a common feature of diseased skin. 242 56

Carpal tunnel syndrome developed concurrently with cutaneous connective tissue disease in five patients. The skin lesions varied from morphea, lichen sclerosus, fasciitis, and discoid lupus erythematosus to lupus panniculitis. Variable and transitory serologic and direct immunofluorescent findings were noted. In two cases, surgical specimens from carpal tunnel operations had lymphoid nodules. Treatment of the cutaneous connective tissue syndrome (antimalarials, four cases; corticosteroids, two cases) brought healing of the carpal tunnel syndrome as well as improvement of the skin lesions.
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PMID:Carpal tunnel syndrome in cutaneous connective tissue disease: generalized morphea, lichen sclerosus, fasciitis, discoid lupus erythematosus, and lupus panniculitis. 710 1

Two patients with lobular lymphohistiocytic panniculitis had chronic lesions that produced subcutaneous atrophy and that were responsive to antimalarial drugs. Massive lymphocytic infiltrate was associated with caseation necrosis of the fat lobules. In both patients, an unusual antibody to extractable nuclear antigen was seen, and antinuclear antibody was sometimes present. The unusual manifestation, course of the disease, serologic findings, and response to therapy suggest that the panniculitis is related to (but does not evolve into) lupus erythematosus panniculitis or subcutaneous morphea or both. A satisfactory term for this entity would be "connective tissue panniculitis."
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PMID:Connective tissue panniculitis. 736 44

A 22-year-old female with morphea-like lesions, deep subcutaneous nodules and lipoatrophic areas of the skin on lateral aspects of the upper arms, on the breasts and on the buttocks is described. In 1990 a biopsy specimen obtained from a subcutaneous nodule showed hyaline necrosis of fat tissue; there were no epidermal changes. Direct immunofluorescence revealed granular deposits of IgM at the dermo-epidermal junction of the skin overlying the subcutaneous nodule. In a biopsy specimen taken at the onset of the disease in 1988, hyaline sclerosis of the deep dermis, follicular hyperkeratosis and vacuolar degeneration in the epidermis were described. There was weak positivity for antinuclear antibodies. The diagnosis of lupus erythematosus panniculitis (LEP) was made. Administration of chloroquine resulted in complete clearing of nodules in 3 months. The reported case demonstrates the difficulties in establishing the diagnosis of LEP in patients who present with subcutaneous disease, morphea-like lesions and who do not have other clinical or laboratory evidence of lupus erythematosus. The differential diagnosis of LEP and deep morphea is discussed.
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PMID:Lupus erythematosus panniculitis with morphea-like lesions. 831 47


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